A Call for Help From A Cystic Fibrosis Patient Directed at the Canadian Government

Marten Devlieger’s sister Karen, a cystic fibrosis patient, died of the disease at the age of thirty-three. According to a recent article in CTV News Canada, Marten was also diagnosed with the fatal disease at two years of age.

Marten has spent many years of fundraising and advocating for cystic fibrosis awareness. But as of the latter part of 2019, when the Vertex Pharmaceutical drug Trikafta was approved in the United States, he changed his focus.

Marten is making every effort to appeal to the provincial and federal governments of Canada for immediate action on behalf of cystic fibrosis patients. Each Canadian province will decide independently whether the drug will be covered. It may be several months before the drug approval process even begins.

According to B.C. PharmaCare, people in charge of providing data to support the efficacy of the drug have in fact provided insufficient evidence. Another factor that B.C. PharmaCare might find problematic is the cost of the drug which is about $300,000 per year.

About Trikafta

 Trikafta is a combination of elexacaftor, tezacaftor, and ivacaftor fixed-dose tablets combined plus ivacaftor tablets for people over the age of twelve. The drug Trikafta is used to treat people with one or more copies of a mutation called F508del the most common CF mutation.

About Cystic Fibrosis (CF)

The DNA “letters” in a gene spell out instructions to make a protein. If the protein is not made correctly, it can lead to a myriad of problems.

This is the case with the genetic disorder cystic fibrosis. CF occurs when the cystic fibrosis transmembrane conductance regulator (CFTR) protein is not made correctly or when it is not made at all. CF causes lung infections and severe lung damage. The eventual result is respiratory failure.

The CFTR gene has over seventeen hundred mutations. CF patients usually have two copies of the CFTR gene, one from each parent.

Approximately 4,300 Canadians have been diagnosed with CF. According to the Cystic Fibrosis Foundation, life expectancy for patients is about fifty-two years.

Marten explained that there are so many CF patients that are extremely ill. He wants to speak out now on their behalf while he is still well enough to get this critical message out to the public and to the government.

 


 

 

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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